Mater Hospital, Mater Centre for Neurosciences, Brisbane, Queensland, Australia.
The University of Queensland, School of Medicine, Brisbane, Queensland, Australia.
Intern Med J. 2022 Nov;52(11):1943-1949. doi: 10.1111/imj.15472. Epub 2022 Jun 23.
Anti-N-methyl-D-aspartate-receptor (anti-NMDA-R) encephalitis is a complex autoimmune neuropsychiatric syndrome. Although initially associated with ovarian teratoma, subsequent studies have demonstrated that anti-NMDA-R encephalitis may occur without an identifiable cause or be triggered by viral infection of the central nervous system such as herpes simplex virus encephalitis (HSVE).
To present details from a Queensland cohort analysing triggering events in patients with anti-NMDA-R encephalitis in an Australian context.
The authors identified patients with anti-NMDA-R encephalitis diagnosed and managed through public hospitals in Queensland, Australia, between 2010 and the end of 2019. Data collected included demographics, clinical presentation, investigation results, management and outcome measurements.
Thirty-one cases of anti-NMDA-R encephalitis were included in the study. Three cases of anti-NMDA-R encephalitis were triggered by prior HSVE, five cases were associated with ovarian teratoma and 23 cases had no identifiable trigger. There were an additional three cases in which anti-NMDA receptor antibodies were present in the context of other disease states but where the patient did not develop anti-NMDA-R encephalitis. Cases triggered by HSVE or associated with ovarian teratoma experienced a more severe disease course compared to cases with no identifiable trigger. All groups responded to immunosuppressive or immunomodulatory therapy. Analysis of clinical characteristics revealed a complex heterogeneous syndrome with some variability between groups.
In this cohort, the number of cases of anti-NMDA-R encephalitis triggered by HSVE is comparable to those triggered by ovarian teratoma. However, the majority of cases of anti-NMDA-R encephalitis had no identifiable trigger or associated disease process.
抗 N-甲基-D-天冬氨酸受体（anti-NMDA-R）脑炎是一种复杂的自身免疫性神经精神综合征。尽管最初与卵巢畸胎瘤有关，但随后的研究表明，抗 NMDA-R 脑炎可能在没有明确病因的情况下发生，也可能由单纯疱疹病毒脑炎（HSVE）等中枢神经系统病毒感染引发。
在澳大利亚背景下，报告昆士兰州队列分析抗 NMDA-R 脑炎患者触发事件的详细信息。
作者确定了 2010 年至 2019 年底期间在澳大利亚昆士兰州公立医院诊断和治疗的抗 NMDA-R 脑炎患者。收集的数据包括人口统计学、临床表现、检查结果、治疗和预后测量。
本研究纳入了 31 例抗 NMDA-R 脑炎患者。3 例抗 NMDA-R 脑炎由先前的 HSVE 引发，5 例与卵巢畸胎瘤有关，23 例无明确诱因。另有 3 例在其他疾病状态下存在抗 NMDA 受体抗体，但患者未发展为抗 NMDA-R 脑炎。由 HSVE 引发或与卵巢畸胎瘤相关的病例与无明确诱因的病例相比，疾病过程更为严重。所有组均对免疫抑制或免疫调节治疗有反应。对临床特征的分析揭示了一种复杂的异质综合征，各组之间存在一定的变异性。
在本队列中，由 HSVE 引发的抗 NMDA-R 脑炎病例数量与由卵巢畸胎瘤引发的病例数量相当。然而，大多数抗 NMDA-R 脑炎病例没有明确的诱因或相关疾病过程。
